The group of restrictive cardiomyopathies (RKMP) includes a number of myocardial and endomyocardial diseases. These entities are characterized by stiffening of both ventricles resulting in a deterioration of ventricular filling and severe diastolic dysfunction. Systolic ventricular function is usually normal or only mildly impaired. Amyloidosis is the most prevalent underlying cause, but also sarcoidosis, hypereosinophilic syndrome with endocardial fibrosis and some inherited metabolic and storage diseases are common. Constrictive pericarditis is the main differential diagnosis, but noninvasive (magnetic resonance imaging, echocardiography, natriuretic peptides) and invasive diagnostic procedures allow separation of these two entities. Diagnosis of the underlying disease is mandatory for therapy of RKMP, as there are specific therapeutic options for symptom relief. Heart transplantation may be an option in end-stage heart failure.